Juxtapapillary capillary hemangiomas - Clinical features and visual acuityoutcomes

Objective: To describe the clinical features, association with von Hippel-L indau (VHL) disease and visual acuity outcomes of patients with a juxtapapi llary capillary hemangioma. Design: Retrospective observational case series. Participants: Seventy-two eyes of 68 patients identified with a juxtapapill ary capillary hemangioma. Follow-up data of at least 6 months duration were available for 60 eyes. Methods: A retrospective chart review of patients diagnosed with a juxtapap illary capillary hemangioma examined at four medical centers. Main Outcome Measures: Age at diagnosis, Visual acuity (VA) at first examin ation and at last follow-up, tumor growth pattern and location, associated clinical features, type of treatment, association with VHL, and presence of peripheral hemangiomas were recorded for each patient. Results: On initial examination, VA was greater than or equal to 20/40 in 4 3 of 70 eyes (61 %) and was greater than or equal to 20/200 in 60 eyes (86% ). At an average follow-up of 5.4 years (range, 0.5-19 years), VA of greate r than or equal to 20/40 was achieved in 21 eyes (35%) and greater than or equal to 20/200 in 33 eyes (55%). Patients with VHL had poorer initial VA ( 48% vs. 70% with VA greater than or equal to 20/40, and 74% vs. 93% with VA greater than or equal to 20/200) and final VA (26% vs. 41% with VA greater than or equal to 20/40, and 39% vs. 65% with VA greater than or equal to 2 0/200) compared with patients without VHL. Patients with VHL more commonly were seen at an earlier age (average, 20 vs. 44 years, P < 0.001), with bil ateral (17% vs. 0%), and/or peripheral (39% vs. 0%) (P < 0.001) tumors that more often had an endophytic growth pattern (63% vs. 22%, P = 0.001) compa red with patients without VHL. Patients selected for laser treatment genera lly had poorer initial (52% vs. 74% with VA greater than or equal to 20/40, 79% vs. 96% with VA greater than or equal to 20/200) and final VAs (18% vs . 56% with VA greater than or equal to 20/40, 45% vs. 67% with VA greater t han or equal to 20/200) compared with patients not treated with laser. Conclusions: On long-term follow-up of patients with a juxtapapillary capil lary hemangioma, the VA generally worsens. Patients with VHL and a juxtapap illary hemangioma more often present at a younger age, have tumors with an endophytic growth pattern, and have bilateral, multiple tumors. Treatment w ith laser photocoagulation results in variable VA outcomes. (C) 2000 by the American Academy of Ophthalmology.